Background-We have previously shown that a specific combination of drug therapy and left ventricular assist device unloading results in significant myocardial recovery, sufficient to allow pump removal, in two thirds of patients with dilated cardiomyopathy receiving a Heartmate I pulsatile device. However, this protocol has not been used with nonpulsatile devices. Methods and Results-We report the results of a prospective study of 20 patients who received a combination of angiotensin-converting enzymes, ␤-blockers, angiotensin II inhibitors, and aldosterone antagonists followed by the ␤ 2 -agonist clenbuterol and were regularly tested (echocardiograms, exercise tests, catheterizations) with the pump at low speed. Before left ventricular assist device insertion, patient age was 35.2Ϯ12.6 years (16 male patients), patients were on 2.0Ϯ0.9 inotropes, 7 (35%) had an intra-aortic balloon pump, 2 were hemofiltered, 2 were ventilated, 3 had a prior Levitronix device, and 1 had extracorporeal membrane oxygenation. Cardiac index was 1.39Ϯ0.43 L ⅐ min Ϫ1 ⅐ m Ϫ2 , pulmonary capillary wedge pressure was 31.5Ϯ5.7 mm Hg, and heart failure history was 3.4Ϯ3.5 years. One patient was lost to follow-up and died after 240 days of support. Of the remaining 19 patients, 12 (63.2%) were explanted after 286Ϯ97 days. Eight had symptomatic heart failure for Յ6 months and 4 for Ͼ6 months (48 to 132 months). Before explantation, at low flow for 15 minutes, ejection fraction was 70Ϯ7%, left ventricular end-diastolic diameter was 48.6Ϯ5.7 mm, left ventricular end-systolic diameter was 32.3Ϯ5.7 mm, mV O 2 was 21.6Ϯ4 mL ⅐ kg Ϫ1 ⅐ min Ϫ1 , pulmonary capillary wedge pressure was 5.9Ϯ4.6 mm Hg, and cardiac index was 3.6Ϯ0.6 L ⅐ min Ϫ1 ⅐ m Ϫ2 . Estimated survival without heart failure recurrence was 83.3% at 1 and 3 years. After a 430.7Ϯ337.1-day follow-up, surviving explants had an ejection fraction of 58.1Ϯ13.8%, left ventricular end-diastolic diameter of 59.0Ϯ9.3 mm, left ventricular end-systolic diameter of 42.0Ϯ10.7 mm, and mV O 2 of 22.6Ϯ5.3 mL ⅐ kg Ϫ1 ⅐ min Ϫ1 . Conclusions-Reversal of end-stage heart failure secondary to nonischemic cardiomyopathy can be achieved in a substantial proportion of patients with nonpulsatile flow through the use of a combination of mechanical and pharmacological therapy. (Circulation. 2011;123:381-390.)

Background-We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. Methods and Results-Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63.6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. Conclusions-Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases. (Circulation. 1999;100[suppl II]:II-29-II-35.) Key Words: aneurysm Ⅲ valves Ⅲ aorta M arfan syndrome is an autosomal dominant heritable disorder of connective tissue that results in skeletal, ocular, and cardiovascular manifestations. The diagnosis, which has been the subject of several publications, 1-5 is made by clinical and genetic criteria. The prognosis in Marfan syndrome is determined mainly by pathological changes in the aortic root and ascending aorta, which frequently involve the aortic valve. Changes in the aortic valve mechanism are due largely to secondary aortic wall disease. To date, there is no agreement on the type and timing of surgical treatment of aortic valve and root disease in Marfan syndrome. Most patients undergo composite replacement of the aortic root and valve with a Bentall procedure. 6-14 However, all available valve substitutes carry a significant incidence of valve-related complications such as hemorrhage, thromboembolism, infection, and compromised hemodynamics. These complications could be particularly relevant in patients with Marfan syndrome because of the young age, possible existence of left ventricular disease, diffuse arterial pathology, tendency to arterial dissection or leakage, and occasional need for orthopedic operations. In 1979, we developed a valve-conserving operation for patients...

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